Hematology

1. Hematology Jan Bazner-Chandler CPNP, CNS, MSN, RN

2. Blood • Blood is the fluid of life • Blood is composed of: • Plasma • RBC • WBC • Platelets

3. Plasma • Plasma consists of: • 90% water. • 10 % solutes: albumin, electrolytes and proteins. • Proteins consist of clotting factors, globulins, circulating antibodies and fibrinogen.

4. Red Blood Cells • RBC’s travel through the body delivering oxygen and removing waste. • RBC’s are red because they contain a protein chemical called hemoglobin which is bright red in color. • Hemoglobin contains iron, making it an excellent vehicle for transporting oxygen and carbon dioxide.

5. Average life cycle is 120 days. The bones are continually producing new cells. RBC’s

6. White Blood Cells • The battling blood cells. • The white blood cells are continually on the look out for signs of disease. • When a germ appears the WBC will: • Produce protective antibodies. • Surround it and devour the bacteria.

7. WBC’s • WBC life span is from a few days to a few weeks. • WBC’s will increase when fighting infection.

8. Platelets are irregularly-shaped, colorless bodies that are present in blood. Their sticky surface lets them form clots to stop bleeding. Platelets

9. Blood Values • CBC with differential and platelet count. • Hgb: • Normal levels are 11 to 16 g / dl • Panic levels are: • Less than 5 g / dl • More than 20 g / dl

10. Hematocrit • Normal hematocrit levels are 35 to 44%. • Panic levels: • Hmct less than 15 % • Hmct greater than 60%

11. Hemoglobin and Hematocrit • Can be used as a simple blood test to screen for anemia. • The CBC with differential would be used to help diagnose a specific disorder. • A bone marrow aspiration would be the most conclusive in determining cause of anemia – aplastic / leukemia.

12. Bone Marrow Bone marrow is the spongy substance found in the center of the bones. • It manufactures bone marrow stem cells, which in turn produce blood cells. • Red blood cells – carry oxygen to tissue • Platelets – help blood to clot • White blood cells – fight infection

13. Bone Marrow Transplant • Donor is placed under anesthesia. • Marrow is aspirated out of the iliac crest. • Marrow is filtered and treated to remove bits of bone and other unwanted cells and debris, transferred to a blood bag, and is infused into the patient’s blood just like at transfusion.

14. Bone Marrow Aspiration

15. Treatment Modalities • Transfusion: • Packed red blood cells – anemia • Platelets – platelet dysfunction • Fresh frozen plasma – coagulation factors

16. Blood Transfusions • 3 types of transfusion reactions • Hemolytic • Allergic • Febrile

17. Hemolytic Reaction • Refers to an immune response against transfused blood cells. • Antigens, on the surface of red blood cells, are recognized as “foreign proteins” and can stimulate B lymphocytes to produce antibodies to the red blood cell antigens.

18. Hemolytic reaction • Flank pain • Fever • Chills • Bloody urine • Rash • Low blood pressure • Dizziness / fainting

19. Nursing Management • Stop the blood transfusion. • Start normal saline infusion. • Take vital signs with blood pressure • Call the MD • Obtain blood sample and urine specimen. • Return blood to blood bank. • Document

20. Febrile Reaction • Often occurs after multiple blood transfusions. • Symptoms:fever, chills, and diaphoresis. • Interventions: • Slow transfusion and administer antipyretic. • Administer antipyretic prior to administration.

21. Allergic Reaction • Symptoms: rash, urticaria, respiratory distress, or anaphylaxis. • Interventions: • administer antihistamine before transfusion • Physician may order washed rbc’s

22. Hematologic Conditions

23. Alteration in Hematologic Status • Disorders of hemostasis or clotting factors • Structural or quantitative abnormalities in the hemoglobin. • Anemias • Aplastic Anemia

24. Genetic Implications • The following have a genetic link: implications for genetic screening and fetal diagnosis • Sickle cell anemia • Thalassemia • Hemophilia

25. Bleeding Disorders • Three types Hemophilia: males only • Type A most common – factor VIII deficiency • Type B - lack of factor IX (Christmas Disease) • Type C – lack of factor XI Von Willebrand Disease – 1% of population – men or women – prolonged bleeding time

26. Hemophilia Type A • Hemophilia type A is the deficiency of clotting factor VIII. • A serious blood disorder • Affects 1 in 10,000 males in the US • Autoimmune disorder with lowered level of clotting factor • All races and socio economic groups affected equally

27. Hemophilia • Hemophilia is a sex-linked hereditary bleeding disorder • Transmitted on the X chromosome • Female is the carrier • Women do not suffer from the disease itself

28. Historical Perspective • First recorded case in Talmud Jewish text by an Arab physician – documentation of two brothers with bleeding after circumcision. • Queen Victoria is carrier and spread the disease through the male English royalty.

29. Goals of Care • Goals of care: • Provide factor VIII (IX) to aid blood in clotting. • To decrease transmission of infectious agents in blood products; hepatitis & AIDS. • Future: gene therapy to increase production of clotting factor.

30. Symptoms • Circumcision may produce prolonged bleeding. • As child matures and becomes more active the incidence of bleeding due to trauma increases

31. Symptoms • May be mild, moderate or severe • Bleeding into joint spaces, hemarthrosis • Most dangerous bleed would be intracranial.

32. Diagnosis • Presenting symptoms • Prolonged activated aPTT and decreased levels of factor VIII or IX. • Genetic testing to identify carriers

33. Treatment • Products used to treat hemophilia are: • Fresh frozen plasma and cryoprecipitate which are from single blood donors and require special freezing. • Second generation of factor VIII are made with animal or human proteins.

34. Nursing Diagnoses • Risk for injury • Pain with bleed especially into a joint • Impaired physical mobility • Knowledge deficit regarding disease and management of disease

35. Nursing interventions • No rectal temps. • Replace the factor as ordered by physician. • Manage pain utilizing analgesics as ordered. • Maintaining joint integrity during acute phase: immobilization, elevation, ice. • Physical therapy to prevent flexion contraction and to strengthen muscles and joints. • Provide opportunities for normal growth and development.

36. Teaching • Avoid aspirin which prolongs bleeding time in people with normal levels of factor VIII. • A fresh bleeding episode can start if the clot becomes dislodged. • Natural reactions in the body cause the clot that is no longer needed to “break down. This process occurs 5 days after the initial clot is formed.

37. Family Education • Medic-Alert bracelet • Injury prevention appropriate for age • Signs and symptoms of internal bleeding or hemarthrosis • Dental checkups • Medication administration

38. Long Term Complications • 20% develop neutralizing antibodies that make replacement products less effective. • Gene therapy providing continuous production of the deficient clotting factor could be the next major advance in hemophilia treatment.

39. Disseminated Intravascular Coagulation or DIC • DIC is an acquired coagulopathy that is characterized by both thrombosis and hemorrhage. • DIC is not a primary disorder but occurs as a result of a variety of alterations in health.

40. Assessment • The most obvious clinical feature of DIC is bleeding. • Renal involvement = hematuria, oliguria, and anuria. • Pulmonary involvement = hemoptysis, tachypnea, dyspnea and chest pain. • Cutaneous involvement = petechiae, ecchymosis, jaundice, acrocyanosis and gangrene.

41. Management of DIC • Treatment of the precipitating disorder. • Supportive care with administration of platelet concentration and fresh frozen plasma and coagulation factors. • Administration of heparin (controversial in children). • Heparin potentates anti-thrombin III which inhibits thrombin and further development of thrombosis.

42. Nursing Diagnoses • Altered tissue perfusion • Risk for injury • Anxiety

43. Nursing Interventions • Rigorous ongoing assessment of all body systems • Monitor bleeding • No rectal temps • Avoid trauma to delicate tissue areas • All injections sites and IV sites need to be treated like an arterial stick.

44. Prognosis • Depends on the underlying disorder and the severity of the DIC.

45. ITP • Idiopathic thrombocytopenic purpura • Idiopathic = cause is unknown • Thrombocytopenic = blood does not have enough platelets • Purpura = excessive bleeding / bruising

46. Immune Thrombocytopenic Purpura • Antibodies destroy platelets • Antibodies see platelets as bacteria and work to eliminate them • ITP is preceded by a viral illness • URI • Varicella / measles vaccine • Mononucleosis • Flu

47. Symptoms • Random purpura • Epistaxis, hematuria, hematemesis, and menorrhagia • Petechiae and hemorrhagic bullae in mouth

48. Diagnostic Tests • Low platelet count • Peripheral blood smear • Antiplatelet antibodies Normal platelet count: 150,000 to 400,000

49. Management • IV gamma globulin to block antibody production, reduce autoimmune problem • Corticosteroids to reduce inflammatory process • IV anti-D to stimulate platelet production

50. Sickle Cell Anemia • Autosomal recessive disorder • Defect in hemoglobin molecule • Cells become sickle shaped and rigid • Lose ability to adapt shape to surroundings. • Sickling may be triggered by fever and emotional or physical stress